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Isolated Epicardial Langerhans Cell Histiocytosis in a Neonate With Complete Spontaneous Regression
Lucas Oliveira Rocha1, Ana Paula Kuczynski Pedro Bom2,3, Milen Minkov4,5
1Department of Pediatric Cardiology, Hospital Pequeno Principe, Curitiba, Brazil.
Insights
We report a rare case of neonatal Langerhans cell histiocytosis (LCH) presenting as an isolated epicardial mass. The mass showed complete spontaneous regression, highlighting conservative management for localized cardiac LCH.
Area of Science:
- Pediatric Cardiology
- Hematology-Oncology
- Rare Diseases
Background:
- Langerhans cell histiocytosis (LCH) is a rare clonal myeloid neoplasm.
- Cardiac involvement in neonates with LCH is exceptionally uncommon.
- Epicardial masses in neonates require careful etiological investigation.
Purpose of the Study:
- To report a unique case of neonatal LCH with isolated epicardial involvement.
- To illustrate the diagnostic and management approach for this rare condition.
- To emphasize the potential for spontaneous regression and conservative management.
Main Methods:
- Multimodal cardiac imaging (echocardiography, MRI) for anatomical assessment.
- Histopathological examination of the epicardial mass with immunohistochemistry (CD1a, CD207, S-100, ALK, BRAFV600E).
- Systemic staging to exclude disseminated disease.
Main Results:
- A term male neonate presented with prenatally detected pericardial effusion and a postnatally identified epicardial mass.
- Biopsy confirmed Langerhans cell histiocytosis (LCH), negative for ALK and BRAFV600E mutations.
- Imaging confirmed the mass was confined to the visceral pericardium without myocardial infiltration.
- The patient remained hemodynamically stable and disease was localized.
- Serial imaging demonstrated complete spontaneous regression of the mass by 6 months.
Conclusions:
- Isolated epicardial LCH in neonates, though rare, can occur.
- Comprehensive staging and precise imaging are crucial for guiding management.
- Conservative observation may be a safe and effective strategy for hemodynamically stable infants with localized epicardial LCH.
Abstract:
Langerhans cell histiocytosis (LCH) is a rare clonal myeloid neoplasm. Cardiac involvement is exceedingly uncommon in neonates. We report a term male neonate with prenatally detected pericardial effusion and a postnatally identified epicardial mass adherent to the left ventricle. Multimodal imaging demonstrated confinement to the visceral pericardium without myocardial infiltration. Biopsy confirmed LCH (CD1a+, CD207+, S-100+, ALK-, BRAFV600E-) and staging excluded systemic disease. The patient remained hemodynamically stable and was managed conservatively after multidisciplinary evaluation. Serial imaging demonstrated complete spontaneous regression by 6 months. Comprehensive staging and precise anatomical imaging can guide safe observation in isolated epicardial LCH.
